Rare neural tube defect separated, successfully repaired on 11-day-old child at GPHC

The surgical team with (INSET) little Shailah Persaud

When Altia Atkinson, a 34-year-old housewife, from Reliance, Essequibo found out that she was pregnant earlier this year, she and her husband Satesh Persaud, a 41-year-old Carpenter, were elated.

Already a mother of five (5) healthy children, Alita anticipated an uncomplicated pregnancy and delivery was inevitable.

As Alita sought out prenatal care at her community Health Centre, her ultrasounds appeared normal and showed no cause for concern. It wasn’t until she was admitted for the delivery of her 6th baby that her ultrasound showed some irregularity – however, physicians at the Suddie Hospital were unable to determine if the mass/growth they were detecting was growing on Alita or her unborn child.

Following a caesarean section, Shailah Ariya Persaud was born on September 19, 2022, at Suddie Hospital and was immediately referred to the Georgetown Public Hospital Corporation (GPHC) on account of a malformation that presented as a second head.

Shailah Persaud with the malformation

Just a few days old, Shailah was reviewed by Chief Neurosurgeon, Dr. Amarnauth Dukhi, who quickly diagnosed little Shailah with a neural tube defect. After MRI scans of the brain and cervical spine was done, Dr. Dukhi confirmed a rare Cranio-Cervical Junction malformation, a combination of the neural tube defects of the brain and spine as one, a Myelomeningocele-encephalocele occurring at the back of the head where the brain joins the spinal cord.

While neural tube defects are quite a common birth defect, they usually occur on the head or the spine separately – in fact, the occurrence of encephalocele and myelomeningocele together as one defect in the same patient is rarely described in medical literature.

In these uncommon cases the neural tube defect presents as sac-like protrusions on the brain and spine and are caused by the failure of the neural tube to close completely during foetal development, which may be attributed to several internal and external factors, mainly lacking the use of Folic Acid during pregnancy.

The management of encephalocele and myelomeningocele is often associated with numerous challenges including blood loss, cardio-respiratory disturbances and hypothermia. Surgical intervention is critical for prevent hemodynamic fluctuations and excessive pressure on the sac which may result in premature rupture and eventual death.

Neurosurgeon Dr Amarnauth Dukhi with Shailah and her parents after the surgery

On the 30th of September, Chief Neurosurgeon, Dr Dukhi led a multi-disciplinary team at GPHC to separate this large malformation and repair the defect at the junction where brain and spinal cord are joined.

This tedious and complicated medical intervention for Shailah commenced firstly with the placement of VP shunt for the developing Hydrocephalus that was also diagnosed. The large malformation/mass, the size of the normal head was then carefully separated from the head and upper cervical spine. This was followed by the reconstruction of the cranio-spinal junction’s defect to prevent the leakage of cerebrospinal fluid and allow for normal development of brain and spinal structures. After the more than six (6) hour procedure, Dr. Dukhi and team deemed it a successful one.

Following seven (7) days of neonatal post-operative care, little Shailah was discharged into her mother’s arm without any obvious neurological deficits and is expected to lead normal childhood. Close monitoring will be done to determine any difficulty in the child’s developmental curve.